Instructive CasesAnakinra And Etanercept Combination Treatment in a Child With Severe, Nonresponsive Kawasaki DiseaseWalser, Matthias*; Hermann, Matthias MD*; Hufnagel, Markus MD†; Haas, Nikolaus A. MD*; Fischer, Marcus MD*; Dalla-Pozza, Robert MD*; Jakob, André MD*Author Information From the *Department of Pediatric Cardiology and Pediatric Intensive Care, Ludwig-Maximilians-University of Munich, Marchioninistr. 15, 81377 Munich, Germany †Division of Pediatric Infectious Disease and Rheumatology, Department of Pediatrics and Adolescent Medicine, University Medical Center, Medical Faculty, University of Freiburg. Mathildenstr. 1, 79106 Freiburg, Germany. Accepted for publication May 28, 2020. The authors have no funding or conflicts of interest to disclose. M.W. collected data, carried out the initial analyses and interpretation, drafted the article, and reviewed and revised the manuscript. M.H. collected data, carried out the initial analyses and interpretation, critically reviewed the manuscript, and revised the manuscript. M.H. contributed to analysis and interpretation of data and reviewed and revised the manuscript critically for important intellectual content. N.A.H., M.F., R.D.-P. served on the clinical patient management team, coordinated and supervised data collection, contributed to analyses and interpretation of data, and critically reviewed the manuscript. A.J. conceptualized and designed the study, drafted the initial manuscript, and reviewed and revised the manuscript. All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work. Address for correspondence: Matthias Walser, Department of Pediatric Cardiology, Ludwig Maximilian-University of Munich, Marchioninistr. 15, D-81377 Munich, Germany. E-mail: [email protected]. The Pediatric Infectious Disease Journal: October 2020 - Volume 39 - Issue 10 - p e310-e313 doi: 10.1097/INF.0000000000002810 Buy Metrics Abstract Kawasaki disease (KD) patients’ resistance to treatment with intravenous immunoglobulins (IVIG) places them at high risk for an unfavorable progression of the disease. In these patients, there has been little evidence that alternative treatments are effective. Nevertheless, biologicals such as an interleukin-1-receptor blocker and tumor-necrosis-factor-α inhibitors increasingly have been used. If the patient does not respond to one of these therapeutics, a combination of 2 biologicals might be an alternative, but this is not yet generally accepted due to the potentially increased risk of infection. Here we report on a 3-month-old boy suffering from severe refractory KD. KD diagnosis was delayed due to the misinterpretation of a urinary tract infection and to the short and nonsimultaneous presence of classical KD symptoms. After complete KD later was able to be diagnosed, treatment with intravenous immunoglobulins was administered. However, the disease proved resistant to 2 courses of IVIG, as well as to corticosteroids. The patient developed giant coronary artery aneurysms early during the course of disease. Anakinra was initiated, but even with stepwise higher anakinra dosages, he remained febrile and coronary artery dimensions increased. Therefore, etanercept was added as a second biological. Only under combination treatment with anakinra and etanercept were his inflammation and fever able to be completely resolved. Coronary artery dimensions improved over time. Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.